Somatostatinoma of the Minor Papilla Treated by Local Excision in a Patient with Neurofibromatosis Type 1

  • Ramesh Bhandari Department of Surgery, University of Melbourne, Austin Health. Heidelberg, Melbourne, Victoria, Australia
  • Georgina Riddiough Department of Surgery, University of Melbourne, Austin Health. Heidelberg, Melbourne, Victoria, Australia
  • Julie Lokan Department of Pathology, University of Melbourne, Austin Health. Heidelberg, Melbourne, Victoria, Australia
  • Laurence Weinberg Department of Anaesthesia, University of Melbourne, Austin Health. Heidelberg, Melbourne, Victoria, Australia
  • Marios Efthymiou Department of Gastroenterology, University of Melbourne, Austin Health. Heidelberg, Melbourne, Victoria, Australia
  • Mehrdad Nikfarjam Department of Surgery, University of Melbourne, Austin Health. Heidelberg, Melbourne, Victoria, Australia
Keywords: Mastectomy, Segmental, Neuroendocrine Tumors, Neurofibromatoses, Pancreatic Ducts


Context Somatostatinoma arising from the minor papilla in a patient with neurofibromatosis type 1 (NF1) is a known but very rare condition, which may cause non-specific symptoms and can present because of its mass effect. Case report A fifty-year-old female presenting with ongoing non-specific abdominal pain for a few months duration was found to have a mass involving the minor papilla. She had a history of NF1 but was otherwise well. Magnetic resonance imaging showed a dilated pancreatic duct and the finding of pancreatic divisum. The lesion was 18fluorine-fluoro-2-deoxyglucose positron emission tomography/computed tomography and 68gallium (Ga) DOTATATE negative. Endoscopic ultrasound revealed a 1.7 cm lesion confined to the minor ampulla. Endoscopic retrograde pancreatography attempts with biopsy and endoscopic ultrasound fine needle aspiration biopsy were inconclusive and resulted in mild pancreatitis on two occasions. Open local excision of the minor papilla was undertaken without complications. Histology confirmed a completely excised grade 1 neuroendocrine tumor with intense diffuse somatostatin staining. Conclusion Somatostatinoma of the minor papilla is a rare tumor that most commonly occurs in the setting of NF1 and may be amenable to local excision.

Image: Pancreatic duct reconstruction.


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Pancreatic duct reconstruction
How to Cite
BhandariR., RiddioughG., LokanJ., WeinbergL., EfthymiouM., & NikfarjamM. (2015). Somatostatinoma of the Minor Papilla Treated by Local Excision in a Patient with Neurofibromatosis Type 1. JOP. Journal of the Pancreas, 16(1), 81-84.